Rabbit anti-Human UFD1L Polyclonal Antibody

Uniprot Id

Q92890

Target Species

Human

Target Name

UFD1

Target Full Name

Ubiquitin recognition factor in ER-associated degradation protein 1

Target Function

Essential component of the ubiquitin-dependent proteolytic pathway which degrades ubiquitin fusion proteins. The ternary complex containing UFD1, VCP and NPLOC4 binds ubiquitinated proteins and is necessary for the export of misfolded proteins from the ER to the cytoplasm, where they are degraded by the proteasome. The NPLOC4-UFD1-VCP complex regulates spindle disassembly at the end of mitosis and is necessary for the formation of a closed nuclear envelope. It may be involved in the development of some ectoderm-derived structures. Acts as a negative regulator of type I interferon production via the complex formed with VCP and NPLOC4, which binds to DDX58/RIG-I and recruits RNF125 to promote ubiquitination and degradation of DDX58/RIG-I.

Target Subcellular Location

Nucleus. Cytoplasm, cytosol.

Target Protein Families

UFD1 family

Target Tissue Specificity

Found in adult heart, skeletal muscle and pancreas, and in fetal liver and kidney.

Target Research Area

Cell Biology

Target Synonyms

UB fusion protein 1; Ubiquitin fusion degradation 1 like (yeast); Ubiquitin fusion degradation 1 like; Ubiquitin fusion degradation protein 1 homolog; UFD1; UFD1_HUMAN; UFD1L

Target Background

The protein encoded by this gene forms a complex with two other proteins, nuclear protein localization-4 and valosin-containing protein, and this complex is necessary for the degradation of ubiquitinated proteins. In addition, this complex controls the disassembly of the mitotic spindle and the formation of a closed nuclear envelope after mitosis. Mutations in this gene have been associated with Catch 22 syndrome as well as cardiac and craniofacial defects. Alternative splicing results in multiple transcript variants encoding different isoforms. A related pseudogene has been identified on chromosome 18.